Morphea: a practical review of its diagnosis, classification and treatment

Morphea, or localized scleroderma, is a rare disease of the connective tissue that manifests itself with localized sclerosis of the skin and, in some cases, with extracutaneous manifestations. Its etiology is not fully understood, but it is believed that there is genetic predisposition, in addition to environmental triggering factors. Classification of the disease is not simple due to its multiple presentations; however, it is useful in order to define the treatment, which should be individualized and started early to avoid cosmetic and functional complications. In this review, we summarize the most important practical aspects of the classification, diagnostic methods and evaluation of morphea activity, as well as available therapeutic options, with an emphasis on existing clinical evidence regarding their efficacy and safety.

Localized scleroderma: clinical spectrum and therapeutic update

Scleroderma is a rare connective tissue disease that is manifested by cutaneous sclerosis and variable systemic involvement. Two categories of scleroderma are known: systemic sclerosis, characterized by cutaneous sclerosis and visceral involvement, and localized scleroderma or morphea which classically presents benign and self-limited evolution and is confined to the skin and/or underlying tissues. Localized scleroderma is a rare disease of unknown etiology. Recent studies show that the localized form may affect internal organs and have variable morbidity. Treatment should be started very early, before complications occur due to the high morbidity of localized scleroderma. In this review, we report the most important aspects and particularities in the treatment of patients diagnosed with localized scleroderma.

MORFEAS: clasificación y tratamiento / MORFEAS: classification and treatment

Las morfeas (esclerodermias localizadas) constituyen un espectro de afecciones cutáneas caracterizadas por endurecimiento por fibrosis exagerada de la dermis y la hipodermis que adoptan típicas aunque diferentes morfologías clínicas, localizaciones, profundidad lesional, número de lesiones, curso evolutivo y pronóstico. Las morfeas comparten además un inicio predominante antes de los 15 años de edad, una gran variedad de autoanticuerpos séricos, potenciales y serias consecuencias funcionales y estéticas. Complicaciones extracutáneas aparecen sólo excepcionalmente, principalmente trastornos encefálicos en los pacientes con morfea cefálica. Un diagnóstico correcto y por tanto el inicio de un manejo terapéutico suelen ocurrir con un infortunado retardo de meses o años. Se han reportado diversas formas de tratamiento, algunas bastante controvertidas, basadas principalmente en experiencias anecdóticas o series limitadas de pacientes. Por otra parte, ha sido difícil validar parámetros confiables para monitorear la actividad, la severidad y la mejoría objetiva de los pacientes. El resultado terapéutico actual es sólo parcialmente exitoso y no hay guías terapéuticas aceptadas por todos. Esta revisión se limita a discutir las dificultades de clasificación de las morfeas, proponer una clasificación personal y revisar las modalidades terapéuticas usadas por los diferentes grupos de dermatólogos y reumatólogos a lo largo de los últimos 25 años

The morpheas (localized sclerodermas) represent a spectrum of cutaneous disorders characterized by skin sclerosis caused by exaggerated dermal and hypodermal fibrosis adopting different although typical clinical morphologies, localizations, lesional depth, number of lesions, course and prognosis. Other aspects shared by the morpheas are an onset mainly in patients before 15 years old, a wide variety of serum autoantibodies and potential serious functional and aesthetic consequences. Extracutaneous complications appear only by exception, mainly encephalic disorders in patients with cephalic morphea. The correct diagnosis and consequently the onset of therapy both usually happen with an unfortunate delay of months or years. Many ways of treatment have been reported, some quite controversial, mainly based in anecdotic experiences or limited series of patients. Furthermore it has been difficult to validate reliable parameters for monitoring the activity, severity and objective improvement of patients. Therapy results are at present only partially successful and there are no widely accepted therapeutic guidelines. This paper is limited to discuss the difficulties in classifying the morpheas, to propose a personal classification and to review the therapeutic modalities used by different groups of dermatologists and rheumatologists through the last 25 years